trophils (7,200/mm3 with 65% neutrophils at admis- sion). Examination of a CSF specimen obtained by lumbar puncture showed 400 leukocytes/mm3 (300 neutrophils and 100 lymphocytes), 480 erythrocytes/
mm3, 7.74 g of protein/L, and 1 mmol glucose/L. Bac- teriological, virological, and mycological studies of the second CSF specimen were started immediately after the lumbar puncture, before the initiation of antibiotic therapy. These studies were negative, as were cultures of blood and urine specimens. Probabilistic antibiotic therapy with ceftriaxone and fosfomycin was started.
The neurological manifestations improved gradually;
on the fourth day; however, a fever spike and exacerba- tion of the headaches prompted a third lumbar punc- ture. The CSF contained 96 leukocytes/mm3, 6400 erythrocytes/mm3, 1.05 g of protein/L, and 3.8 mmol of glucose/L; the C-reactive protein level was 35 mg/L.
The antibiotics were stopped on the eighth day upon reception of the negative results from all the microbio- logical studies; at the time, the patient was free of symptoms. Two months later, his body temperature was normal, he had no symptoms other than the nerve root pain that had persisted unchanged since the injec- tion, and his C-reactive protein level was normal.
DISCUSSION
The onset of the symptoms within the first few hours after the injection and the negative results of the micro- biological studies conducted under optimal conditions strongly suggest aseptic meningitis. Although similar cases have been reported [2], the CSF changes were different from those in our patient. In the case reported by Karmochkine et al., the protein level was 2.7 g/L, the glucose level 1.3 mmol/L, and the leukocyte count 12,700/mm3 (87% neutrophils). In our patient, although the cell count was unimpressive, the protein level was extremely high (more than 7 g/L), suggesting severe acute inflammation of the meninges.
Methylprednisolone in a polyethylene glycol vehicle (Depomedrolt) was responsible for the first instances of glucocorticoid-induced meningeal reaction. This preparation has been the main source of such reactions and has been reported to cause necrosis of the axons and myelin [5]. Our patient received hydrocortisone acetate, a compound better known for its ability to induce seizures [5]. Furthermore, the dose was 50 mg, as com- pared to 125 mg in earlier cases [4].
The outcome of glucocorticoid-induced meningeal reactions is usually rapidly and spontaneously favorable
[3]. Cytological and, above all, microbiological studies of the CSF should be performed, and probabilistic treatment with two antibiotics given until the results of the cultures are available [2].
REFERENCES
1 Dougherty JH, Richard JR, Fraser AR. Complications follow- ing intraspinal injections of steroids. J Neurosurg 1978 ; 48 : 1023-5.
2 Karmochkine M, Chaibi P, Rogeaux O, Koeger AC, Bour- geois P. Méningite chimique simulant une méningite infec- tieuse après infiltration intradurale de corticoïdes. Presse Méd 1993 ; 22 : 82.
3 Plumb VJ, Dismukes WE. Chemical meningitis related to imtrathecal corticosteroid therapy. South Med J 1977 ; 70 : 1241-3.
4 Hoeffel C, Gaucher H, Chevrot A, Hoeffel JC. Complication of lumbar puncture with injection of hydrosoluble material J.
Spinal Disord 1999 ; 12 : 168-71.
5 Bernat JL. Intraspinal steroid therapy. Neurology 1981 ; 31 : 168-71.
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Ankylosing spondylitis and Behçet’s disease in
combination.Two case reports
Noufissa Etaouil, Elouarda Benyahya, Rajae Bennis, Ouafa Mkinsi
Service de Rhumatologie, CHU Ibn Rochd, Casablanca, Morocco
The peripheral joint manifestations of Behçet’s disease are common and well known. In contrast, the occur- rence of Behçet’s disease and ankylosing spondylitis in the same patient is rare [1]. We report two new cases.
CASE 1
This 34-year-old man was admitted in 1994 for a 3-year history of inflammatory pain in the spine and heels with redness of the eyes. He reported recurrent oral ulcerations for the last 10 years, without genital ulcerations. Stiffness of the spine, necrotic pseudofolli- culitis of the back and thighs, and hypersensitivity at injection sites were the main physical findings. Bilateral panuveitis with retinal vasculitis in the right eye were noted by the ophthalmologist. The erythrocyte sedi- mentation rate was 100 mm/h. Tests were positive for
96 Letters to the Editor
HLA B27 and negative for HLA B5. Radiographs showed sacroiliitis with obliteration of the joint space on both sides. The spine was radiographically normal.
The ocular manifestations responded to corticosteroid and cyclophosphamide therapy. Nonsteroidal anti- inflammatory drugs and colchicine were given also, to good effect on the mucocutaneous, spinal, and periph- eral joint manifestations. Several recurrences of uveitis were noted. After 2 years, thoracolumbar syndesmo- phytes and bilateral calcaneal enthesitis developed.
CASE 2
A 27-year-old man was admitted in 1997 for a 4-year history of hip pain, alternating buttock pain, and inflammatory low back pain, with recurrent oral ulcer- ations and genital ulcerations during the last year.
Motion range was limited at the lumbar spine and hips.
Two ulcerations were visible on the tongue and one on the scrotum. The pathergy test was positive. Findings were normal from an ophthalmological examination.
The erythrocyte sedimentation rate was 80 mm/h and the C-reactive protein level was 28 g/L. The patient was HLA B27-positive and HLA B5-negative. Radiographs disclosed bilateral stage III sacroiliitis and bilateral nar- rowing of the coxofemoral joint space, with no spinal abnormalities. No further progression occurred with nonsteroidal anti-inflammatory drugs, colchicine, glu- cocorticoid injections into the hips, sulfasalazine, and physical therapy.
DISCUSSION
Joint involvement occurs in 45 to 70% of patients with Behçet’s disease, usually manifesting as arthralgia and as acute or subacute arthritis [1, 2]. Although concomi- tant occurrence of Behçet’s disease and spondyloarthr- opathy is infrequent, the number of reports is increasing, inviting questions about the nosology of these condi- tions. Both our patients had definite diagnoses of Behçet’s disease and ankylosing spondylitis. The first patient (case 1) met three of the major criteria of Masson and Barnes for Behçet’s disease; major criteria 1, 3, and 4 of Hamza;, and the criteria of the interna- tional study group on Behçet’s disease. This patient had no genital ulcerations, a symptom absent in 20% of patients with Behçet’s disease [1]. The second patient (case 2) satisfied three major criteria of Masson and Barnes, criteria 1, 2, and 4 of Hamza; and the criteria of the international study group on Behçet’s disease [1, 3].
The total number of points in the Amor’s criteria set for
spondyloarthropathy [4] was 11 (without counting the uveitis) in the first patient and 10 in the second patient;
both patients met the criteria of the European Spondy- loarthropathy Study Group.
Although the diagnosis of these two conditions is often easy, as in our patients, the potential link between them remains unclear. It has been suggested that chance alone may be involved [2, 5], but some studies found that patients with one disorder often had manifesta- tions of the other, with for instance sacroiliitis and spondyloarthropathy in 35% and 10% of 182 Turks with Behçet’s disease studied by Dilsen et al. [6].
Overall, nearly 90% of spondyloarthropathy patients are positive for HLA B27 and 60–80% of Behçet’s disease patients are positive for HLA B5 [1, 7]. How- ever, patients with both Behçet’s disease and spondy- loarthropathy are far less likely to be HLA B5-positive than those with Behçet’s disease only [1, 8]. Neither of our patients had the HLA B5 antigen. Furthermore, HLA B27 does not seem more common in Behçet’s disease than in the population at large [1, 9]. However, the prevalence of HLA B27 is extremely high among patients with both Behçet’s disease and spondyloarthr- opathy [1, 10]. Hamza et al. suggested that Behçet’s disease may increase the risk of spondyloarthropathy among patients with the HLA B27 antigen [1].
Although both our patients had HLA B27, only case 1 supports Hamza’s hypothesis, with a seven-year inter- val between the onset of Behçet’s disease and the sub- sequent onset of ankylosing spondylitis. The negative correlation between ankylosing spondylitis and Behçet’s disease in patients such as ours and the substantial proportion of Behçet’s disease patients who are negative for HLA B5 could also suggest the opposite effect (case 2).
CONCLUSION
The combination of Behçet’s disease and spondyloar- thropathy is uncommon but probably involves more than chance alone. Ascertainment of all cases of this combination, with full HLA testing in each patient, would help to generate pathophysiological hypotheses.
REFERENCES
1 Hamza M, Ayed K, Zribi A. Maladie de Behçet. In: Kahn MF, Peltier A, Meyer O, Piette JC, Eds. Maladies systémiques. Paris:
Flammarion; 1991. p. 917-47.
2 Benamour S. Manifestations rhumatismales de la maladie de Behçet. Ann Méd Interne (Paris) 1999 ; 150 : 562-70.
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3 Masson RM, Barnes CG. Behcet’s disease with arthritis. Ann Rheum Dis 1969 ; 28 : 95.
4 Amor B. Les spondylarthropathies. Rhumatologie. 2e ed.. Paris:
Maloine; 1990. p. 94-107.
5 Yurdakul S, Yazici H, Tuzun Y. The joint involvement in Behçet disease [abstract]. Rev Rhum Mal Ostéoartic 1981 ; 48 (Suppl) : 0293.
6 Dilsen N, Konice M, Aral O. Why Behçet disease should be accepted as a seronegative arthritis. In: Lehner T, Barner C, Eds.
Recent advances in Behçet disease. International Congress and Symposium series. n °103. London, New York: Royal Society of Medicine services; 1966. p. 281-4.
7 Kallel MH, Bejai, Fournié B, Fournié A. Behçet syndrome with ankylosing spondylitis. Rev Rhum [Engl Ed] 1995 ; 62 : 295-9.
8 Hamza M, Ayed K, Hamza S. Association spondylarthrite ankylosante et maladie de Behçet (4 cas): étude du système HLA [résumé]. Rev Rhum Mal Ostéoartic 1981 ; 48 : 6.
9 O’Duffy JD, Tswell HF, Elueback LR. HLA antigens in Beh- cet’s disease. J Rheumatol 1976 ; 3 : 1-3.
10 Dubost JJ, Sauverzie B, Galtier B, Bussière JL, Rampon S.
Syndrome de Behçet et spondylarthrite ankylosante. Rev Rhum Mal Ostéoartic 1985 ; 52 : 457-61.
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